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肥厚型心肌病梗阻型-左心室流出道梯度:激发的新方法、生物标志物监测及治疗的最新进展

Obstructive Form of Hypertrophic Cardiomyopathy-Left Ventricular Outflow Tract Gradient: Novel Methods of Provocation, Monitoring of Biomarkers, and Recent Advances in the Treatment.

作者信息

Dimitrow Pawel Petkow, Rajtar-Salwa Renata

机构信息

2nd Department of Cardiology, Jagiellonian University Collegium Medicum, Kopernika Street 17, 31-501 Cracow, Poland.

出版信息

Biomed Res Int. 2016;2016:1575130. doi: 10.1155/2016/1575130. Epub 2016 May 10.

Abstract

Dynamic (latent or/and labile) obstruction of left ventricular outflow (LVOT) was recognized from the earliest clinical descriptions of hypertrophic cardiomyopathy (HCM) and has proved to be a complex phenomenon, as well as arguably the most audible ("visible") pathophysiological hallmark of this heterogeneous disease. The aim of the current review is focused on two novel issues in a subgroup of obstructive HCM. Firstly, the important methodological problem in HCM is the examination of a subgroup of patients with nonobstructive hypertrophy in resting conditions and hard, but possible provoking obstruction. Recently, investigators have proposed physiological stress test (with double combined stimuli) to disclose such type of patients. The upright exercise is described in the ESC guideline on hypertrophic cardiomyopathy from 2014 and may appear as a candidate for gold standard provocation test. The second novel area of interest is associated with elevated level of signaling biomarkers: hypercoagulation, hemolysis, acquired von Willebrand 2A disease, and enhanced oxidative stress. The accelerated and turbulent flow within narrow LVOT may be responsible for these biochemical disturbances. The most recent advances in the treatment of obstructive HCM are related to nonpharmacological methods of LVOT gradient reduction. This report extensively discusses novel methods.

摘要

左心室流出道(LVOT)的动态(潜在或/和不稳定)梗阻自肥厚型心肌病(HCM)最早的临床描述中就已被认识到,并且已被证明是一种复杂的现象,也可以说是这种异质性疾病最明显(“可见”)的病理生理特征。本综述的目的聚焦于梗阻性HCM亚组中的两个新问题。首先,HCM中的一个重要方法学问题是对静息状态下无梗阻性肥厚但在用力时可能诱发梗阻的患者亚组进行检查。最近,研究人员提出了生理应激试验(采用双重联合刺激)来识别这类患者。2014年欧洲心脏病学会(ESC)肥厚型心肌病指南中描述了直立运动,它可能成为金标准激发试验的候选方法。第二个新的关注领域与信号生物标志物水平升高有关:高凝状态、溶血、获得性血管性血友病2A型疾病以及氧化应激增强。狭窄LVOT内加速和紊乱的血流可能是这些生化紊乱的原因。梗阻性HCM治疗的最新进展与降低LVOT压差的非药物方法有关。本报告广泛讨论了新方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc4e/4877458/f48f58ab629a/BMRI2016-1575130.001.jpg

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